Biliary cystadenoma and cystadenocarcinoma pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Suveenkrishna Pothuru, M.B,B.S. [2]

Overview

On gross pathology, biliary cystadenoma is unilocular or multilocular cystic lesion filled with fluid or blood. Cystic fluid may be clear and mucinous. On microscopic histopathological analysis, presence of biliary-type epithelium wall supported by ovarian-like stroma is the characteristic finding of biliary cystadenoma.

Pathophysiology

The vast majority of biliary cystadenomas are intrahepatic (97%) with a small proportion extrahepatic (3%). Only rarely are they found in the extrahepatic biliary tree and gallbladder.

Gross Pathology

• Biliary cystadenomas are cystic neoplasms that may be either unilocular or multilocular.
• The septa within the cyst may rarely show calcification.
• The majority of biliary cystadenomas do not communicate with the bile ducts, but luminal communication may be occasionally observed.
• The cystic fluid may be clear and mucinous.
• Blood stained fluid within the cyst indicates a malignant component (cystadenocarcinoma).
• Rarely, the fluid may be bile stained, purulent, proteinaceous, or gelatinous.

Microscopic Pathology

Biliary cystadenoma is usually a multiloculated cyst covered by a biliary-type epithelium wall and surrounded by an ovarian-like stroma, containing smooth muscle cells. Microscopic evaluation can easily distinguish cystadenoma from cystadenocarcinoma, based on nuclear pleomorphism and loss of nuclear stratification in the latter.

• Biliary cystadenomas are composed of multiple cysts lined by single layer of cuboidal or nonciliated columnar epithelium that resembles normal biliary epithelium resting on a basement membrane.
• At places the epithelium forms multiple polypoidal or papillary projections.
• In some of the cases, dysplastic mucinous epithelium itself may proliferate within the bile ducts causing obstruction. This variant is considered an intraductal papillary neoplasm with prominent cystic dilatation of the duct rather than a true biliary cystic neoplasm.
• The ovarian-like stroma is thick consisting of compact spindle-shaped cells and supports the epithelium and is often observed exclusively in women.
• A histological variant of biliary cystadenomas has been described, namely cystadenomas with mesenchymal stroma. This variant, which is more common in females, is characterized by the presence of spindle cells in the mesenchymal stroma. These cells are capable of differentiating into different cell types, with a high premalignant potential.
• The tumor may express receptors for progesterone while histological characteristics include positivity for vimentin and cytokeratin.

References

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